Aeglea BioTherapeutics to Present New Phase 1/2 Trial Results in Arginase 1 Deficiency at the 2018 ACMG Annual Clinical Genetics Meeting

AUSTIN, Texas, April 03, 2018 -- Aeglea BioTherapeutics, Inc. (NASDAQ:AGLE), a clinical-stage biotechnology company that designs and develops innovative human enzyme therapeutics for patients with rare genetic diseases and cancer, today announced that it will present new data on Arginase 1 Deficiency patients at the 2018 Annual Clinical Genetics Meeting of the American College of Medical Genetics and Genomics (ACMG) in Charlotte, North Carolina on Thursday, April 12. The new data will include additional clinical insights on short-term treatment with repeat doses of pegzilarginase, including baseline standardized assessments of neuromotor function. The Company will conduct a clinical update conference call at 8:30 a.m. ET on April 12.

Title: Weekly Pegzilarginase Produces Marked and Sustained Reductions in Guanidino Compounds in Adults with Arginase 1 Deficiency: Early Phase 1/2 Results

Presenting Author: Roberto Zori, M.D., University of Florida, Gainesville, FL

Poster Number: 803

Presentation Date and Time: Thursday, April 12, 10:00 a.m. to 11:30 a.m. ET

An electronic version of the presentation will be available for download from the Presentations & Events section of the Company’s investor relations website beginning on the day of the their presentation at the 2018 ACMG Annual Clinical Genetics Meeting.

Conference Call & Webcast Details
Aeglea will hold a clinical update conference call on Thursday, April 12, 2018 at 8:30 a.m. ET. To access the live conference call via phone, please dial 1-877-709-8155 (toll free) within the United States, or 1-201-689-8881 internationally. A replay of the call will be available through April 19, 2018 by dialing 1-877-660-6853 within the United States or 1-201-612-7415 internationally. The conference ID is 13678293.

To access the live and archived webcast of the presentation, please visit the Presentations & Events section of the Aeglea BioTherapeutics investor relations website. Please connect to the website at least 15 minutes prior to the presentation to allow for any software download that may be necessary.

About Pegzilarginase (AEB1102) in Arginase 1 Deficiency
Pegzilarginase is an enhanced human arginase that enzymatically degrades the amino acid arginine. Aeglea is developing pegzilarginase for the treatment of patients with Arginase 1 Deficiency, a debilitating urea cycle disorder caused by deficiency of a key arginine metabolizing enzyme that leads to severe and progressive hyperargininemia-related neurological abnormalities, hyperammonemia and early mortality. Pegzilarginase is intended for use as an enzyme replacement therapy in patients to reduce elevated blood arginine levels. The Company’s Phase 1 data demonstrated that pegzilarginase reduced blood arginine levels into the normal range, supporting its mechanism of action.

About Aeglea BioTherapeutics
Aeglea is a clinical-stage biotechnology company that designs and develops innovative human enzyme therapeutics for patients with rare genetic diseases and cancer. The Company is developing pegzilarginase, its lead investigational therapy, for the treatment of Arginase 1 Deficiency, as monotherapy in arginine-dependent cancers and in combination with an immune checkpoint inhibitor for small cell lung cancer. In addition, Aeglea has an active research pipeline of other human enzyme-based approaches in both therapeutic areas. For more information, please visit http://aegleabio.com.

Safe Harbor / Forward Looking Statements
This press release contains "forward-looking" statements within the meaning of the safe harbor provisions of the U.S. Private Securities Litigation Reform Act of 1995. Forward-looking statements can be identified by words such as: "anticipate," "intend," "plan," "goal," "seek," "believe," "project," "estimate," "expect," "strategy," "future," "likely," "may," "should," "will" and similar references to future periods. These statements are subject to numerous risks and uncertainties that could cause actual results to differ materially from what we expect. Examples of forward-looking statements include, among others, the timing and success of our clinical trials and related data, the timing of announcements and updates relating to our clinical trials and related data, our ability to enroll patients into our clinical trials, success in our collaborations and the potential therapeutic benefits and economic value of our lead product candidate or other product candidates. Further information on potential risk factors that could affect our business and its financial results are detailed in our most recent Annual Report on Form 10-K for the year ended December 31, 2017 filed with the Securities and Exchange Commission (SEC), and other reports as filed with the SEC. We undertake no obligation to publicly update any forward-looking statement, whether written or oral, that may be made from time to time, whether as a result of new information, future developments or otherwise.

Media Contact:
David Calusdian
Sharon Merrill Associates
617.542.5300
[email protected]

Investor Contact:
Charles N. York II
Chief Financial Officer
Aeglea BioTherapeutics
[email protected]